inovmedИнновационная медицина КубаниInnovative Medicine of Kuban2541-9897Scientific Research Institute – Ochapovsky Regional Clinical Hospital No. 110.35401/2541-9897-2023-8-4-142-147inovmed-773Research ArticleОБЗОРЫREVIEWSТаргетная терапия в лечении муковисцидоза у детейTargeted Therapy for Cystic Fibrosis in Childrenhttps://orcid.org/0000-0002-9653-6365БурлуцкаяА. В.BurlutskayaA. V.

Бурлуцкая Алла Владимировна, д. м. н., заведующая кафедрой педиатрии № 2

Краснодар

Alla V. Burlutskaya, Dr. Sci. (Med.), Head of Pediatrics Department No. 2

Krasnodar

https://orcid.org/0009-0008-4947-5660БрисинВ. Ю.BrisinV. Yu.

Брисин Виктор Юрьевич, врач-педиатр, пульмонолог, заведующий пульмонологическим отделением

Краснодар

Viktor Yu. Brisin, Pediatrician, Pulmonologist, Head of the Pulmonology Division

Krasnodar

https://orcid.org/0000-0003-3632-1386СтатоваА. В.StatovaA. V.

Статова Анастасия Васильевна, к. м. н., доцент кафедры педиатрии № 2

Краснодар

Anastasia V. Statova, Cand. Sci. (Med.), Associate Professor at Pediatrics Department No. 2

Krasnodar

https://orcid.org/0009-0001-4834-6655ЗенкинаО. Ю.ZenkinaO. Yu.

Зенкина Оксана Юрьевна, ассистент кафедры педиатрии № 2

Краснодар

Oksana Yu. Zenkina, Assistant Professor at Pediatrics Department No. 2

Krasnodar

https://orcid.org/0000-0002-0674-7194ПисоцкаяЮ. В.PisotskayaJ. V.

Писоцкая Юлия Васильевна, ординатор кафедры педиатрии № 2

350063, Краснодар, ул. М. Седина, 4

Julia V. Pisotskaya, Resident, Pediatrics Department No. 2

ulitsa M. Sedina 4, Krasnodar, 350063

ms.pisotskaya2017@gmail.comКубанский государственный медицинский университетРоссияKuban State Medical UniversityRussian FederationДетская краевая клиническая больницаРоссияChildren’s Regional Clinical HospitalRussian Federation20233012202304142147Copyright © Бурлуцкая А.В., Брисин В.Ю., Статова А.В., Зенкина О.Ю., Писоцкая Ю.В., 20232023Бурлуцкая А.В., Брисин В.Ю., Статова А.В., Зенкина О.Ю., Писоцкая Ю.В.Burlutskaya A.V., Brisin V.Y., Statova A.V., Zenkina O.Y., Pisotskaya J.V.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.innovmedkub.ru/jour/article/view/773

Мутация в гене CFTR нарушает работу одноименного белка, который располагается на поверхности апикальной мембраны эпителиальных клеток и функционирует как хлорный канал. Открытие патогенетических лекарств – CFTR­-модуляторов является достижением в терапии муковисцидоза.

Цель работы: Анализ современных научных работ отечественных и зарубежных авторов, посвященных таргетной терапии муковисцидоза у детей. В статье также освещены историческая справка о муковисцидозе, эволюция в диагностике и лечении данной патологии, механизмы воздействия таргетной терапии при лечении муковисцидоза.

A mutation in the CFTR gene causes the malfunctioning of the CFTR protein that is located on the apical membrane of epithelial cells and functions as a chloride channel. The discovery of CFTR modulators is an advance in cystic fibrosis treatment.

Objective: To analyze modern Russian and foreign literature on targeted therapy for cystic fibrosis in children. We also describe the history of cystic fibrosis, evolution in its diagnosis and treatment, and mechanisms behind targeted therapy for cystic fibrosis.

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The authors declare that there are no conflicts of interest present.